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Long-term outcome in Fontan procedure patients: the destination therapy or a bridge to transplant?
Session:
Posters (Sessão 3 - Écran 1) - Cardiopatias Congénitas no Adulto
Speaker:
Miguel Martins de Carvalho
Congress:
CPC 2022
Topic:
F. Valvular, Myocardial, Pericardial, Pulmonary, Congenital Heart Disease
Theme:
20. Congenital Heart Disease and Pediatric Cardiology
Subtheme:
20.2 Congenital Heart Disease – Epidemiology, Prognosis, Outcome
Session Type:
Pósters Electrónicos
FP Number:
---
Authors:
Miguel Martins de Carvalho; Ricardo Alves Pinto; Tânia Proença; Catarina Costa; Filipa Amador; Joao Calvao; Catarina Marques; Andre Cabrita; Maria Cristina Cruz; Filipe Macedo
Abstract
<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><span style="font-size:12.0pt">The Fontan procedure (FP) is considered a palliative surgical technique used for complex congenital heart disease (CHD) patients not suitable for biventricular repair. Currently, these patients’ life expectation is extended but they experience high morbidity and mortality risks and their long-term management is challenging. Our aim was to evaluate the morbidity of these patients after a long-term follow-up.</span></span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><span style="font-size:12.0pt">We collected a retrospective cohort of patients palliated with FP, that were followed in an adult CHD outpatient clinic born between 1980 and 2001. </span><span style="font-size:12.0pt">Clinical and echocardiographic data were collected. A time to adverse event analyses was performed.</span></span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><span style="font-size:12.0pt">A total of 26 patients were enrolled, with a median follow-up of 17 years. The median age was 24 (IQR 22-30) year-old, 35% were female. As for the cardiovascular risk factors, none had hypertension or dyslipidaemia, 3,9% had diabetes and 11,5% were smokers or previous smokers. Only 3,9% of patients had chronic kidney disease. Of note, the mean BNP was of 19.4 pg/mL. The main anatomic abnormalities that lead to the FP were single ventricle (53,8%), followed by pulmonary atresia (23,1%), double outlet right ventricle (11,5%), ventricular septal defect (3,9%) and Ebstein anomaly (7,7%). The majority of patients were previously submitted to a shunt (42,3% with a Blalock-Thomas-Taussig and 19,3% with an atrial septostomy); the most prevalent surgical technique was the cavopulmonary connection (69,2% extracardiac, 15,4% intracardiac), followed by the atriopulmonary anastomosis (11,5%). The systemic ventricle was morphologically left in 84,6%. A fenestration or a residual shunt persisted in 30,8%. The mean basal oxygen saturation was of 95%, with 3,9% of patients being cyanotic. The majority of patients were asymptomatic (69,2%), with a normal ventricular function in those with a systemic left-ventricle (91,7% of patients), and a moderately impaired in those with a systemic right-ventricle (50% of patients); more than moderate AV valve regurgitation was present in 7,7%. Pulmonary hypertension was observed in 7,7%. Atrial arrythmias were present in 11,5%. Liver disease affected 50% of patients (1 patient with an hepatocarcinoma) and protein-losing enteropathy was present in 7,7%. One patient was submitted to a re-FP, due to a Fontan circulation obstruction; another patient was submitted to a heart transplant. Regarding the time-to-adverse-events analyses, more than 65% of patients were event-free during the first 15 years of FP. However, after 20 years of FP more than 60% of patients presented with an adverse event. </span></span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><span style="font-size:12.0pt">Survival in FP patients has improved and most of them live without significant morbidity for several years. However, after 20 years of the FP, the majority of patients experience adverse events and even death. Heart transplant is a possible solution for these patients and should start to be considered when adverse events arise.</span></span></span></p>
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